title: Distress in partners of individuals diagnosed with or at high risk of developing tumors due to rare hereditary cancer syndromes.
abstract: OBJECTIVE
  Li Fraumeni syndrome (LFS) and Von Hippel-Lindau disease (VHL) are two rare hereditary tumor syndromes, characterized by a high risk of developing multiple tumors at various sites and ages for which preventive and treatment options are limited. For partners, it may be difficult to deal with the on-going threat of tumors in both their spouse and children. Therefore, this study aims to evaluate the prevalence of and factors associated with psychological distress among partners of individuals with or at high risk of LFS or VHL.
  
  
  METHODS
  As part of a nationwide, cross-sectional study, partners of individuals diagnosed with or at high risk of LFS or VHL were invited to complete a self-report questionnaire assessing distress, worries, and health-related quality of life.
  
  
  RESULTS
  Fifty-five (58%) of those high-risk individuals with a partner consented to having their partner approached for the study. In total, 50 partners (91%) completed the questionnaire, of whom 28% reported clinically relevant levels of syndrome-related distress. Levels of distress and worries of the partners and their high-risk spouse were significantly correlated. Younger age and a lack of social support were also associated significantly with heightened levels of distress and worries. The majority of partners (76%) believed that professional psychosocial support should be routinely offered to them.
  
  
  CONCLUSIONS
  Approximately one-quarter of the partners exhibit clinically relevant levels of distress that warrant psychological support. The distress levels of the 'patient' could potentially be used to identify partners at risk of developing clinically relevant levels of distress.
authors.affiliation: The Netherlands Cancer Institute-Antoni van Leeuwenhoek Hospital, Division of Psychosocial Research and Epidemiology, Amsterdam, The Netherlands.
authors.forename: C R M
authors.initials: CR
authors.lastname: Lammens
authors.forename: E M A
authors.initials: EM
authors.lastname: Bleiker
authors.forename: S
authors.initials: S
authors.lastname: Verhoef
authors.forename: M G E M
authors.initials: MG
authors.lastname: Ausems
authors.forename: D
authors.initials: D
authors.lastname: Majoor-Krakauer
authors.forename: R H
authors.initials: RH
authors.lastname: Sijmons
authors.forename: F J
authors.initials: FJ
authors.lastname: Hes
authors.forename: E B
authors.initials: EB
authors.lastname: Gómez-García
authors.forename: T A M
authors.initials: TA
authors.lastname: Van Os
authors.forename: L
authors.initials: L
authors.lastname: Spruijt
authors.forename: R B
authors.initials: RB
authors.lastname: van der Luijt
authors.forename: A M W
authors.initials: AM
authors.lastname: van den Ouweland
authors.forename: M W G
authors.initials: MW
authors.lastname: Ruijs
authors.forename: C
authors.initials: C
authors.lastname: Gundy
authors.forename: T
authors.initials: T
authors.lastname: Nagtegaal
authors.forename: N K
authors.initials: NK
authors.lastname: Aaronson
mesh_terms: D000223:Adaptation, Psychological; D000275:Adjustment Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology*; D000328:Adult; D000368:Aged; D001008:Anxiety Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology*; D003430:Cross-Sectional Studies; D003866:Depressive Disorder / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics / Q000523:psychology*; D006801:Humans; D016864:Li-Fraumeni Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000523:psychology*; D008297:Male; D008875:Middle Aged; D018454:Spouses / Q000523:psychology*; D011795:Surveys and Questionnaires; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000175:diagnosis* / Q000235:genetics / Q000523:psychology*